Neuroimaging of Narcolepsy and Kleine-Levin Syndrome
This article reviews structural and functional brain imaging findings in narcolepsy and Kleine-Levin syndrome. Based on the current state of research, brain imaging is a useful tool to investigate and understand the neuroanatomic correlates and brain abnormalities of narcolepsy and other hypersomnia. (Source: Sleep Medicine Clinics)
Source: Sleep Medicine Clinics - June 13, 2017 Category: Sleep Medicine Authors: Seung Bong Hong Source Type: research

The “Known Unknowns” of Kleine-Levin Syndrome
Kleine-Levin syndrome (KLS) is a rare, homogeneous, debilitating sleep disorder characterized by episodic hypersomnia, cognitive impairment, and behavioral changes. The etiology, pathophysiology, and optimal management of KLS remain uncertain. We identify the 5 key areas requiring urgent attention: KLS immunopathogenesis studies, next-generation genetics, multimodal functional imaging, biomarker discovery, and clinical drug trials. A centralized registry of afflicted individuals must be established. Disease uniformity should make the identification of associated genetic or imaging biomarkers easier, but clinical efforts re...
Source: Sleep Medicine Clinics - June 7, 2017 Category: Sleep Medicine Authors: Saad M. Al Suwayri, Ahmed S. BaHammam Source Type: research

Dopamine and serotonin levels in cerebrospinal fluid during episodes of Kleine-Levin syndrome
Kleine-Levin syndrome (KLS) is characterized by recurrent episodes of hypersomnia, cognitive disturbances, apathy, derealization, mood disturbances, hyperphagia, hypersexuality and dysautonomia [1]. The etiology of KLS is unknown. Increased dopamine turnover in the cerebrospinal fluid (CSF) was shown in one patient with KLS and was greater in symptomatic than in the asymptomatic period [2]. The dopamine transporter availability decreased in the striatum of a teenager with KLS and was more marked in the symptomatic than in the asymptomatic phase [3]. (Source: Sleep Medicine)
Source: Sleep Medicine - May 27, 2017 Category: Sleep Medicine Authors: Jean-Baptiste Maranci, Emmanuel Roze, Jean-Fran çois Benoist, Fanny Mochel, Odile Rigal, I. Arnulf Tags: Letter to the Editor Source Type: research

Dopamine and serotonin levels in cerebrospinal fluid during episodes of  Kleine-Levin syndrome
Kleine-Levin syndrome (KLS) is characterized by recurrent episodes of hypersomnia, cognitive disturbances, apathy, derealization, mood disturbances, hyperphagia, hypersexuality and dysautonomia [1]. The etiology of KLS is unknown. Increased dopamine turnover in the cerebrospinal fluid (CSF) was shown in one patient with KLS and was greater in symptomatic than in the asymptomatic period [2]. The dopamine transporter availability decreased in the striatum of a teenager with KLS and was more marked in the symptomatic than in the asymptomatic phase [3]. (Source: Sleep Medicine)
Source: Sleep Medicine - May 27, 2017 Category: Sleep Medicine Authors: Jean-Baptiste Maranci, Emmanuel Roze, Jean-Fran çois Benoist, Fanny Mochel, Odile Rigal, Isabelle Arnulf Tags: Letter to the Editor Source Type: research

Kleine –Levin syndrome; An update and mini-review
Since 1962, when Critchley and Hoffman coined the term Kleine –Levin Syndrome (KLS) for the triad of hypersomnia, excessive eating and “often abnormal behavior” which they have observed in 11 adolescent boys, the number of patients recognized with this rare syndrome expanded, the spectrum of the clinical presentation, disease course, prognosis, gender sp ecificity and the presence of familial cases were established. However, in spite of the progress made in neuroscience, the search for the cause, neuroanatomy, pathophysiology and drug treatment of KLS is still ongoing. (Source: Brain and Development)
Source: Brain and Development - April 20, 2017 Category: Neurology Authors: Natan Gadoth, Arie Oksenberg Tags: Review article Source Type: research

FDG Brain PET/CT Revealing Bilateral Thalamostriatal Activation During a Symptomatic Episode in a Patient With Kleine-Levin Syndrome
We report the case of a 14-year-old girl with clinically diagnosed Kleine-Levin syndrome. Electroencephalogram and brain MRI were unremarkable. An FDG PET/CT scan was acquired during a symptomatic episode, while she was asleep, and another PET scan was obtained when she was asymptomatic, awake, and quiet. Comparison of both examinations demonstrated a bilateral activation of thalami, caudate nuclei, and lenticular nuclei. These findings may be linked to the implication of thalamostriatal structures in the regulation of sleep and wakefulness. (Source: Clinical Nuclear Medicine)
Source: Clinical Nuclear Medicine - April 6, 2017 Category: Nuclear Medicine Tags: Interesting Images Source Type: research

[Kleine-Levin syndrome: differential diagnosis in recurrent encephalitic syndromes in adolescence].
CONCLUSIONS: The Kleine-Levin syndrome presents neurologic symptoms initially more frequently than psychiatric ones. Hypersomnia and behavioural disturbances during wakefulness, bradypsychia, apatheia and emotional liability make us suspect that it could be an encephalitis process. We should be aware of this entity if we face a patient with recurrent encephalitis of unknown origin. PMID: 28345736 [PubMed - in process] (Source: Revista de Neurologia)
Source: Revista de Neurologia - March 29, 2017 Category: Neurology Authors: Duat-Rodriguez A, Martinez-Albadalejo I, Perez-Sebastian I, Cantarin-Extremera V, Hedrera-Fernandez A, Garcia-Penas JJ Tags: Rev Neurol Source Type: research

Kleine-Levin Syndrome
Kleine-Levin syndrome (KLS) is a rare disorder characterized by discrete episodes of hypersomnia associated with cognitive and behavioural abnormalities, as well as normal alertness and function between episodes. The prevalence of KLS may be underestimated as it is often misdiagnosed and managed as another sleep disorder, neurological disorder or psychiatric condition. KLS is more typically seen in adolescence than at other ages, and is more common in males than in females. There are currently neither standard biomarkers nor specific imaging study findings, making the diagnosis of KLS a challenge. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - December 22, 2016 Category: Respiratory Medicine Authors: Olufunke Afolabi-Brown, Thornton B.A. Mason Tags: Mini-Symposium: Pediatric Hypersomnolence Symposium Source Type: research

Migraine in kleine-levin syndrome
Conclusions The association with migraine deserves further investigation, not only as a reliable risk marker but for exploration of the pathophysiology of KLS and novel therapeutic avenues. Initial use of triptans in two of our patients has yielded encouraging results. We encourage others to look for signs of migraine in their patients and consider the use of appropriate therapy. (Source: Journal of Neurology, Neurosurgery and Psychiatry)
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Donnelly, H., Leschziner, G., Marin, J., Nesbitt, A. Tags: Dementia, Drugs: CNS (not psychiatric), Headache (including migraine), Pain (neurology), Sleep disorders (neurology), Ophthalmology, Memory disorders (psychiatry), Sleep disorders ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Prevalence and Mimics of Kleine-Levin Syndrome: A Survey in French-Speaking Switzerland
Conclusions:This study suggests a relatively higher KLS prevalence than previously reported. As compared to KLS, mimic patients have higher age at symptom onset, are more often initially referred for KLS suspicion, and have a higher prevalence of psychiatric disorders.Citation:Habra O, Heinzer R, Haba-Rubio J, Rossetti AO. Prevalence and mimics of Kleine-Levin syndrome: a survey in French-speaking Switzerland.J Clin Sleep Med 2016;12(8):1083–1087. (Source: Journal of Clinical Sleep Medicine : JCSM)
Source: Journal of Clinical Sleep Medicine : JCSM - August 13, 2016 Category: Sleep Medicine Source Type: research

Familial Kleine-Levin Syndrome: A Specific Entity?
Conclusion: < /h3 > < p id="d7e206" > Familial KLS is mostly present in the same generation, and is clinically similar to but slightly less severe than sporadic KLS. < /p > < /div > < div class="section" > < a name="d7e208" > < !-- named anchor -- > < /a > < h3 class="section-title" > Citation: < /h3 > < p id="d7e211" > Nguyen QT, Groos E, Leclair-Visonneau L, Monaca-Charley C, Rico T, Farber N, Mignot E, Arnulf I. Familial Kleine-Levin syndrome: a specific entity? < i > SLEEP < /i > 2016;39(8):1535 & #8211;1542. < /p > < /div > < /div > (Source: Sleep)
Source: Sleep - July 30, 2016 Category: Sleep Medicine Source Type: research

Migraine with brainstem aura presenting as recurrent hypersomnia (Kleine-Levin syndrome).
We describe two patients referred to a tertiary sleep neurology service with a putative diagnosis of Kleine-Levin syndrome. Each described attacks of hypersomnia with elements of migraine with brainstem aura, in addition to having a history of migraine with aura. Simple acute migraine treatment clearly attenuated further attacks. These cases generate discussion as to the common features and potential mechanisms underlying both disorders. Furthermore, they highlight a hitherto underexplored alternative diagnosis of Kleine-Levin syndrome. This provides scope for offering established and effective migraine treatment options t...
Source: Practical Neurology - June 23, 2016 Category: Neurology Authors: Nesbitt AD, Leschziner GD Tags: Pract Neurol Source Type: research

New hypothesis on pontine-frontal eye field connectivity in Kleine-Levin syndrome.
Abstract Previous studies have indicated involvement of the thalamus and the pons in Kleine-Levin syndrome. In the present study, functional connectivity of the thalamus and the pons was investigated in asymptomatic patients with Kleine-Levin syndrome and healthy controls. Twelve patients and 14 healthy controls were investigated by functional magnetic resonance imaging during rest. Resting state images were analysed using seed regions of interest in the thalamus and the pons. The results showed significantly lower functional connectivity between the pons and the frontal eye field in persons with Kleine-Le...
Source: Journal of Sleep Research - May 26, 2016 Category: Sleep Medicine Authors: Engström M, Landtblom AM, Karlsson T Tags: J Sleep Res Source Type: research

Pharmacological treatment for Kleine-Levin syndrome.
CONCLUSIONS: Therapeutic trials of pharmacological treatment for Kleine-Levin syndrome with a double-blind, placebo-controlled design are needed. PMID: 27153153 [PubMed - as supplied by publisher] (Source: Cochrane Database of Systematic Reviews)
Source: Cochrane Database of Systematic Reviews - May 5, 2016 Category: Journals (General) Authors: de Oliveira MM, Conti C, Prado GF Tags: Cochrane Database Syst Rev Source Type: research

Kleine-Levin Syndrome
This article reviews common presenting symptoms, differential diagnosis, diagnostic workup, and potential treatment options. Current updates on functional imaging studies and long-term neuropsychological studies are reviewed. (Source: Current Neurology and Neuroscience Reports)
Source: Current Neurology and Neuroscience Reports - May 1, 2016 Category: Neuroscience Source Type: research